卷 15, 编号 4 (2024)

Neonatal sepsis is one of the most severe diseases of the neonatal period with a high probability of developing multiple organ dysfunction, septic shock and an unfavorable outcome. The purpose of this publication is to present draft federal clinical guidelines for the treatment of sepsis in newborns, which were developed taking into account the principles of evidence-based medicine. During the preparation of clinical guidelines, key questions were formulated using the PIPOH formula: P (population) — patient population and disease characteristics (neonate with sepsis and septic shock); I (interventions) — intensive care measures; P (professionals) — target audience of clinical recommendations (anesthesiologists, intensive care physicians, neonatologists, pediatricians, surgeons, infectious disease doctors); O (outcomes) — expected outcomes that can be improved (patient outcomes, health system indicators, epidemiological indicators, etc.); H (health care setting) — place of medical care (outpatient clinic, hospital, intensive care unit). The project includes sections on the etiology, pathogenesis and diagnosis of sepsis in newborns. Particular attention is paid to the issues of microbiological diagnostics, identification of the pathogen, antibacterial therapy, respiratory and nutritional support, extracorporeal hemocorrection, the use of intravenous immunoglobulins. It has been demonstrated that one of the mandatory elements of therapy is the early initiation of enteral nutrition using breast milk, the use of which is one of the most effective methods for the prevention of late sepsis in premature newborns with low and extremely low birth weight.

BACKGROUND: Currently autologic reconstructive surgery in the most significant approach in surgical therapy for enteral insufficiency due to short bowel syndrome. Possible application for short bowel surgery of widespread urologic Yang–Monti–Casale technique is an issue of interest. The technique includes cutting of small bowel segment antimesenteric border with a figured incision and second tubularization at an angle to the mesenterium fixation line which results in elongation and narrowing of the bowel segment.

AIM: To determine geometric requirements of small bowel segment for Yang–Monti–Casale procedure to treat short bowel syndrome.

MATERIALS AND METHODS: A mathematical model for resulting bowel segment after Yang–Monti–Casale procedure geometry dependency on original bowel length and width was observed. An object of the analysis was a cylindrical surface. Modeling assumptions were that the suface had no thickness and was unstretchable. Only surfaces with length more than width were taken into account. For establishing the dependency of bowel segment after procedure on original parameters a net of cutted with figured incise according the Yang–Monti–Casale technique cylindrical surface was considered. Bowel segment resulting width was computed as a half of the distance between the points that matched during the enteroplasty. Resulting length was computed as the ratio of the surface area and the resulting width.

RESULTS: Yang–Monti–Casale procedure provides significant bowel segment elongation and narrowing if original bowel length to width ratio is not more than 2,5:1. The target bowel width 20 ± 2 mm is achieveable if original length is 60–80 mm and original width is 30–65 mm.

CONCLUSIONS: Yang–Monti–Casale procedure may be considered as auxiliary technique for short bowel syndrome requiring bowel segment length 60–80 mm, width 30–65 mm, and length to width ratio not more than 2,5:1.

Diabetic ketoacidosis is one of the most common complications in children suffering from diabetes mellitus, a complication that may cause cerebral edema. After a diabetic ketoacidosis episode, some children develop cognitive impairment, which could be linked to subclinical or clinical grade cerebral edema. An analysis of scientific literature has shown that the pathophysiological mechanisms of the development of cognitive dysfunction in children with diabetic ketoacidosis-associated cerebral edema have not been fully elucidated. The key pathogenetic hypotheses under consideration include hypoxia, cerebral hypoperfusion and reperfusion, release of vasoactive substances and induction of inflammation, acute hyperglycemia and oxidative stress. Hypoxia leads to hypoxic-ischaemic injury of the brain, affecting, among others, areas responsible for memory formation. Cerebral hypoperfusion and reperfusion trigger mitochondrial dysfunction and oxidative stress, which worsens the cognitive impairment in patients with cerebral edema. Release of vasoactive substances and induction of inflammation impair the functioning of the blood-brain barrier and lead to cerebral vasospasm. Acute hypoglycemia triggers the development of brain tissue acidosis and accumulation of lactate and glutamate, aggravating the cognitive dysfunction. Oxidative stress causes neuronal damage, exacerbating the manifestations of cerebral edema. The pathophysiological mechanisms described here may cause cognitive dysfunction in the setting of cerebral edema either as individual triggers or collectively through a synergistic effect. Further research is needed to expand the knowledge of pathophysiological interconnections between cognitive dysfunction and cerebral edema in children after a diabetic ketoacidosis episode.

Macroamylasemia is a rare disorder characterized by the formation of an amylase-globulin complex that is too large to be easily cleared by the kidneys, resulting in elevated serum amylase levels. It is a benign condition with no serious signs or symptoms and requires no treatment. This article presents a case of long-term asymptomatic hyperamylasemia in a 10-year-old child. The child was admitted to the gastroenterology department with complaints of abdominal pain. Since the age of 8, there have been unexplained increases in serum amylase to 235 U/L and pancreatic amylase to 63.5 U/L, without clinical manifestations of pancreatitis, with normal levels of urine amylase and coprological elastase. An examination was performed, during which pancreatic pathology was excluded. The ratio of amylase and creatinine clearances was 0.754%. This ratio turned out to be less than 1%, which is more likely to indicate macroamysalemia. After further examination, type 1 macroamylasemia was diagnosed, which was the cause of hyperamylasemia — a non-life-threatening biochemical anomaly that requires a detailed examination, broad differential diagnostics, but does not require treatment. However, macroamylasemia is a diagnostic challenge because it must be differentiated from other causes of hyperamylasemia to avoid unnecessary testing and treatment. Hyperamylasemia is one of the main diagnostic criteria for pancreatitis, requiring extensive additional testing and treatment, often in a hospital setting. Therefore, it is important that physicians can recognize and diagnose macroamylasemia at an early stage to avoid unnecessary diagnostic and therapeutic measures. In this regard, we hope that the clinical example we have provided will be interesting and useful for physicians of various specialties.

Anemia of chronic patients is a secondary hematological syndrome, and most often accompanies infectious-inflammatory, immunopathological and tumor processes. The introduction discusses the importance of blood testing to assess the response of the immune and hematopoietic systems to various adverse effects. Various mechanisms are involved in the pathogenesis of anemia, among which the main place belongs to the action of proinflammatory cytokines. In many cases, sideropenia develops that is not associated with an absolute iron deficiency, but occurs as a result of the redistribution of iron reserves in the body. In some patients, diagnosis of various types of pathology begins with the detection of anemia. This requires a comprehensive examination of patients, the exclusion of primary hematological pathology and the detection of all concomitant diseases, including foci of infection. This article presents a clinical example of a multidisciplinary approach to the diagnosis of normochromic normocytic anemia, which resulted in the detection of endocrine pathology — hypoparathyroidism, and chronic foci of Epstein–Barr virus and streptococcal infection in a teenage girl. The peculiarity of the case was that the patient initially consulted a hematologist about mild anemia, but during the examination it became obvious that complaints of progressive weakness, fatigue, hand tremors, and dizziness could not be linked to the existing hematological pathology. Spastic disorders and tetany of the limb muscles that arose during the examination period became the reason for diagnosing metabolic disorders: determining creatine phosphokinase, serum myoglobin, blood electrolytes, including calcium and phosphorus, as well as hormones of the thyroid and parathyroid glands. The examination results showed that the severity of the girl’s condition and neurological symptoms were caused by severe metabolic changes, phosphorus-calcium metabolism disorders in the form of hypocalcemia, hyperphosphatemia, increasing levels of creatine phosphokinase, high levels of lactate dehydrogenase, and were due to endocrine pathology. The presence of neurological symptoms, changes in magnetic resonance imaging and electrocardiogram against the background of a significant decrease in parathyroid hormone, hypocalcemia, and increasing levels of creatine phosphokinase required the patient to be referred to the hospital with a diagnosis of “Hypoparathyroidism”. The presented clinical case confirms the need for an individual, multidisciplinary approach to the diagnosis of secondary anemic conditions, which will allow identifying pathology of various organs and systems.

The article presents a draft of clinical recommendations for the diagnosis and treatment of Erb palsy in birth injury. These recommendations are intended for physicians of maternity hospitals and are aimed at providing information support for decision-making by a doctor in the event of a birth injury, as well as improving the quality of medical care to a patient. The recommendations correspond to the latest scientific data on the topic, and also contain information that is of an applied nature for practical activities. These practical recommendations are offered for public discussion and are posted in full on the website of the Ministry of Health of the Russian Federation. The recommendations contain an information note on Erb palsy in birth injury, including the definition, etiology and pathogenesis of the disease. Three types of classification of generic traumatic lesions of the brachial plexus are presented: by prevalence, by severity of nerve damage and by severity of paresis. The clinical features of the disease are considered in detail. To diagnose the disease, an algorithm of physical examination is proposed, and the use of instrumental research methods: MRI, ultrasonic, X-ray and electromyographic studies. Recommendations for the conservative treatment of pathology and the use of physiotherapy and rehabilitation techniques are given. An algorithm of the doctor’s actions and an information certificate about the disease are proposed to inform the patient’s parents. The recommendations are given taking into account the level of credibility of the recommendations and the level of reliability of the evidence.

Endocrinology, one of the most dynamically developing branches of medicine, has existed as a separate discipline for a little over 100 years. The author of one of the first guidelines on endocrinology in Russia and the founder of the Leningrad School of Clinical Endocrinology was Professor Vasily G. Baranov (1899–1988). In 1926, an endocrinology department was opened at the Department of Therapy of the Leningrad State Institute for Advanced Medical Training under the leadership of Vasily Gavrilovich. Later, in 1963, Professor V.G. Baranov founded the Department of Endocrinology at this educational institution, which currently bears the name of the scientist. V.G. Baranov paid special attention to the issues of endocrine diseases in childhood. Together with academician A.F. Tur, he initiated the creation of an endocrinological service in pediatrics. It was only in 1998 that a new specialty “pediatric endocrinology” was added to the already existing training programs for doctors in the specialty “endocrinology”, opened by order of the Ministry of Health of the Russian Federation. However, the development and formation of pediatric endocrinology as a separate medical discipline took place before official recognition. A special role in the history of this formation belongs to the doctors of St. Petersburg.